Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome
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Synopsis

Patients usually present in the fifth or sixth decade of life, although the onset of symptoms is variable and may take over a decade to develop the classic triad of bilateral vestibulopathy, cerebellar ataxia, and sensory neuropathy. Most patients will have a slowly progressive course. Hearing is typically preserved.
Codes
ICD10CM:G11.2 – Late-onset cerebellar ataxia
G11.9 – Hereditary ataxia, unspecified
SNOMEDCT:
193165008 – Neuropathy in association with hereditary ataxia
95814001 – Peripheral vestibular disease
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Friedreich ataxia
- Spinocerebellar ataxia type 1, 2, 3, 6, or 7
- Multiple system atrophy (cerebellar type)
- Kearns-Sayre syndrome
- Wernicke encephalopathy
- Autosomal dominant sensory ataxia
- Gerstmann-Straussler-Scheinker disease
- Superficial siderosis of the central nervous system
- Paraneoplastic syndrome
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Therapy
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References
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Last Reviewed:10/05/2021
Last Updated:01/12/2022
Last Updated:01/12/2022